以Addison’s病首诊的肾上腺淋巴瘤临床特点分析——附5例报告并文献复习

Clinical features of adrenal lymphoma presenting as Addison＇s disease： 5 case report and literature review

目的：肾上腺淋巴瘤在临床上少见，以Addison’s病首诊者更少见，本文拟探讨以Addison’s病首诊的肾上腺淋巴瘤临床特征、诊治要点及预后，以提高临床医师对其认识。方法回顾性地分析解放军总医院1995年至2014年间收治的以Addison’s病首诊并经病理确诊的5例肾上腺淋巴瘤患者病例资料，统计其症状、体征、病程、肿瘤大小、病理类型及实验室检查结果，并结合国内外文献进行分析。结果5例均为老年患者，年龄（66.4±6.6）岁，男女比例为4∶1。5例患者均有典型Addison’s病的表现，如皮肤变黑、消瘦、乏力、食欲减退。均诊断为原发性肾上腺非霍奇金淋巴瘤，病理类型为B细胞来源，4例明确为弥漫大B细胞型。术后给予R-CHOP方案化疗，其中2例目前健在，发病至今已分别存活了10个月和20个月，随访效果尚可，1例存活3年，余2例存活时间均不足1年。结论以Addison’s病首诊的肾上腺淋巴瘤多发生于老年男性患者，双侧肾上腺受累多见，确诊需依赖穿刺病理诊断，病理类型主要为弥漫大B细胞型，预后较差。

Objective Adrenal lymphoma is a rare entity in clinical practice, especially when presenting as Addison’s disease. Hence, we summarized the clinical features and outcomes of this rare disease in order to raise awareness in clinicians. Methods Clinical data of 5 cases of adrenal lymphoma admitted to our hospital from 1995 to 2014 were retrospectively analyzed. All of them were firstly diagnosed as Addison’s disease and then identified as adrenal lymphoma according to the results of pathology. Their signs, symptoms, tumor size, pathological types and results of laboratory examinations were analyzed, considering literatures review. Results The 5 patients were at an age of （66.4±6.6） years, with a male-to-female ratio of 4∶1. All of them manifested as typical signs and symptoms of Addison’s disease, such as systemic pigmentation, body mass loss, general fatigue and anorexia. They were diagnosed as primary adrenal non-Hodgkin’s lymphoma, with histological type of B-cell origin. Four of them were diagnosed with primary adrenal diffuse large B-cell lymphoma. R-CHOP chemotherapy was administered to all of them, but 2 died within 1 year, 1 died in 3 years, and only 2 survived till now. Conclusion Adrenal lymphoma presenting as Addison’s disease is more common in older males, and usually presents with bilateral adrenal glands. Ultrasound-or CT-guided needle biopsy of the adrenal tumor is necessary to make a definitive histopathological diagnosis. The main histological type is primary adrenal diffuse large B-cell lymphoma, and the prognosis is poor.